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Pulmonary langerhans cell histiocytosis

Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular...

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Published in:Orphanet journal of rare diseases 2012-03, Vol.7 (1), p.16-16, Article 16
Main Authors: Suri, Harpreet S, Yi, Eunhee S, Nowakowski, Gregorz S, Vassallo, Robert
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description Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.
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subjects Adult
Bronchiolitis
Care and treatment
Chemotherapy
Child, Preschool
Cigarette smoke
Cigarettes
Development and progression
Diagnosis
Disease
Female
Health aspects
Histiocytosis, Langerhans-Cell - complications
Histiocytosis, Langerhans-Cell - epidemiology
Histiocytosis, Langerhans-Cell - immunology
Histiocytosis, Langerhans-Cell - pathology
Humans
Hypertension
Hypertension, Pulmonary - complications
Infant
Interstitial
Langerhans cells
Langerhans Cells - immunology
Langerhans Cells - pathology
Langerhans-cell histiocytosis
Lung diseases
Lung Diseases - complications
Lung Diseases - epidemiology
Lung Diseases - immunology
Lung Diseases - pathology
Male
Medical research
Pathogenesis
Pneumothorax - complications
Prognosis
Pulmonary hypertension
Rare diseases
Recruitment
Respiratory Function Tests
Review
Risk factors
Smokers
Smoking
Smoking - adverse effects
Tobacco
Young Adult
title Pulmonary langerhans cell histiocytosis
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